Bickerstaff brainstem encephalitis with or without anti-GQ1b antibody
Keisuke Yoshikawa1, Motoi Kuwahara1, Miyuki Morikawa1
1From the Department of Neurology, Kindai University Faculty of Medicine, Osaka, Japan.
Neurology(R) Neuroimmunology & Neuroinflammation
|September 16, 2020
Summary
Bickerstaff brainstem encephalitis (BBE) with anti-GQ1b antibody presents distinct clinical features, including more frequent upper respiratory infections and sensory disturbances. Intravenous immunoglobulin (IVIG) is a common treatment for these cases in Japan.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Bickerstaff brainstem encephalitis (BBE) is a rare neurological disorder.
- Anti-GQ1b antibodies are implicated in some cases of BBE.
Purpose of the Study:
- To elucidate the clinical distinctions between anti-GQ1b antibody-positive and antibody-negative BBE.
- To characterize the typical presentation and treatment of anti-GQ1b antibody-positive BBE in Japan.
Main Methods:
- A comparative study involving 73 anti-GQ1b antibody-positive BBE cases and 10 antibody-negative BBE cases.
- Clinical data and serum samples were collected from Japanese hospitals (2014-2017).
- Anti-GQ1b antibody presence was confirmed using ELISA.
Main Results:
- Anti-GQ1b antibody-positive BBE cases showed a higher incidence of upper respiratory tract infections and sensory disturbances.
- Cerebrospinal fluid (CSF) analysis revealed lower cell counts and protein levels in antibody-positive cases.
- Brain MRI abnormalities were less frequent, and consciousness disturbance resolved faster in antibody-positive BBE.
- Intravenous immunoglobulin (IVIG) was administered more frequently to anti-GQ1b antibody-positive BBE patients.
Conclusions:
- BBE associated with anti-GQ1b antibodies exhibits a relatively homogeneous clinical profile.
- IVIG is the predominant therapeutic approach for anti-GQ1b antibody-positive BBE in Japan.
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